RHINOSCLEROMA

RHINOSCLEROMA


RHINOSCLEROMA 

  • Progressive granulomatous disease of the upper respiratory tract.
  • Caused by the gram-negative coccobacillus K rhinoscleromatis or Frish Bacillus.

The disease probably begins in areas of epithelial transition such as :

  1. the vestibule of the nose
  2. the subglottic area of the larynx
  3. or the area between the nasopharynx and oropharynx. 
  • It is a chronic infection of the nose characterised by sclerosis and stenosis of the nasal passage.
  • Rhinoscleroma may be found in all age groups, but typically young adults 20–30 years old are most frequently affected.
  • Both sexes are equally affected.
  • The disease is endemic in several parts of world.
  • In India, it is seen more often in northern than in the southern parts.
  • Airborne transmission combined with poor hygiene, crowded living conditions, and poor nutrition contributes to its spread.

PATHOLOGY OF RHINOSLEROMA

  • The disease is characterized by presence of granulations which infiltrate the submucosa ,with scattered:
  1. Mikulicz cells (large vacuolated foamy cells containing a central nucleus and acid fast Frisch bacilli),
  2. Rusell bodies (resmbling plasma cells with an eccentric nucleus and deep eosin staining cytoplasm) and
  3. lymphocytes in dense fibrous tissue.

CLINICAL FEATURES IN A CASE OF RHINOSCLEROMA

SYMPTOMS

  • Blocking of the nose,Nasal Discharge,Headache,Epistaxis

SIGNS

  • External nose is firm and woody to touch.
  • Anterior Rhinoscopy:Throat:may show extension of the sclerosing process downwards from the nose to the oropharynx,larynx.
  • 3 stages:

1. Atrophic Stage: is present initially, with crusting , roomy cavity and atrophic dry nasal mucosa.

2. Granulomatous stage:

  • Granulomatous nodules forms in nasal mucosa.
  • There is also subdermal infiltration of lower part of external nose & upper lip giving a ‘woody feel’.
  • Nodules are painless & non-ulcerative.

3. Cicatricial stage: 

  • Causes stenosis of nares, distortion of upper lip, adhesions in the nose, nasopharynx & oropharynx.
  • May be subglottic stenosis with respiratory distress.
BIOPSY 
  • Shows infiltration of submucosa with plasma cells, lymphocytes, eosinophils, Mikulicz cells & Russell bodies.
  • The latter two are the diagnostic features of the disease. 

TREATMENT IN A CASE OF RHINOSCLEROMA

  • In rhinoscleromatosis, organism may be difficult to eradicate, despite aggressive therapy. Relapses may occur.
  • A combination of conservative surgical debridement and long-term antibiotic coverage is the mainstay of therapy for rhinoscleroma.
  • Tetracycline has been shown to be effective .
  • Fluoroquinolones may be used as an alternative, given their excellent gram-negative activity and convenient dosing regimen 

Exam Important

  • Mikulicz cells and Russell bodies are seen in Rhinoscleroma.
  • A roomy nasal cavity and atrophic dry nasal mucosa with thick crust formation and woody hard external nose is seen in Rhinoscleroma.
  • Rhinoscleroma is caused by Klebsiella Rhinoscleromatis.
  • Rhinoscleroma is a slowly progressing granulomatous disease.
  • Rhinoscleroma is most common in young adults 20–30 years old.
  • Poor nutrition contributes to the spread of Rhinosleroma.
  • Drug of choice in Rhinoscleromatosis is Tetracycline.
  • Frish bacillus causes Rhinoscleroma.
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